Cirrhosis Demo

Cirrhosis Demo

Background of cirrhosis

Type	Clinical features
Definition	Progressive hepatic fibrosis with distorted architecture and regenerative nodules
Etiologies	Hepatitis B and C Alcoholic liver disease Nonalcoholic liver disease Hemochromatosis Autoimmune hepatitis Primary biliary cholangitis (PBC) Primary sclerosing cholangitis (PSC) Medications (eg, methotrexate) Wilson disease Celiac disease Alpha-1 antitrypsin disease Polycystic liver disease Infections (eg, brucella) Right sided congestive heart failure (Cardiac cirrhosis) Polycystic liver disease Veno-occlusive disease
Stages of liver disease	Stages of liver disease Fatty liver Hepatic fibrosis Cirrhosis

Clinical presentation of cirrhosis

Type	Clinical Features
Possible Symptoms	*Compensated* Can be asymptomatic Anorexia Weight loss Weakness and/or fatigue *Decompensated* Pruritus Jaundice GI bleeding (hematemesis, melena, hematochezia) Abdominal distention (ascites) Confusion (hepatic encephalopathy) Muscle cramps Fatigue Lower extremity edema Weight changes Diarrhea Pruritus Amenorrhea in women Hypogonadism in men (eg, erectile dysfunction)
Possible physical exam findings	*Skin* Jaundice Spider angioma on trunk, face, and upper limbs *Head and neck* Icterus in eyes Enlarged parotid gland Fetor hepaticus (sweet pungent breath) Chest Gynecomastia *Abdomen* Hepatomegaly Can also be small or normal size depending on stage of liver disease Splenomegaly Ascites Abdominal distention, fluid wave, dullness to percussion Caput Medusa Portal hypertension causes dilation of periumbilical veins in shape of Medusa’s head Cruveilhier - Baumgarten murmur Venous hum heard best over the epigastric area due to collateral connections between portal vein and remnant of umbilical vein *Extremities* Palmar erythema Changes in nails Muehrcke nails: horizontal white bands Terry nails: white proximal 2/3 of nail plate, red distal 1/3 Clubbing Hypertrophic osteoarthropathy Dupuytren’s contracture *Others* Asterixis in hepatic encephalopathy Testicular atrophy
Possible laboratory/ imaging findings	*Laboratory findings suggesting cirrhosis* Elevated AST > ALT in 2:1 ratio as cirrhosis develops Elevated bilirubin and GGT Decreased albumin Elevated PT/INR Hyponatremia with disease progression Anemia, leukopenia, and thrombocytopenia *Imaging findings suggesting cirrhosis* Ultrasound Can show small and nodular liver, increased echogenicity, and irregular appearing areas Evidence of portal hypertension, ascites, and portal vein thrombosis Elastography Increased stiffness of the tissue
Complications of cirrhosis	Ascites Hepatic encephalopathy Hepatocellular carcinoma Portal hypertensive gastropathy Portal vein thrombosis Umbilical hernia *Variceal bleeding* Gastric, esophageal, splenic, rectal *Spontaneous bacterial peritonitis (SBP)* Positive ascites fluid for bacterial growth Elevated ascitic WBC > 250 cells/mm³ *Hepatorenal syndrome* Renal failure in patients with advanced cirrhosis *Hepatic hydrothorax* Pleural effusion (typically right sided) in cirrhosis without underlying cardiac or pulmonary disease *Hepatopulmonary syndrome* Liver disease Increased alveolar-arterial gradient while breathing room air Evidence for intrapulmonary vascular abnormalities (intrapulmonary vascular dilatations) *Porto-pulmonary hypertension* Pulmonary hypertension with portal hypertension

Diagnosis of cirrhosis

Type	Recommendations
Clinical clues	Stigmata of liver disease on physical examination Laboratory studies suggesting cirrhosis Development of complication of cirrhosis Imaging suggesting cirrhosis
Liver biopsy	Gold standard for diagnosing cirrhosis However, not necessary if clinical clues are consistent with likely cirrhosis
Determine etiology of cirrhosis	Alcohol history with AST/ALT > 2 Hepatitis B and C testing Antimitochondrial antibodies for suspected PBC ANA, ANCA, cholangiography for PSC ANA, Anti-smooth muscle antibodies, ANCA for suspected autoimmune hepatitis Transferrin, plasma ferritin, and gene testing for hemochromatosis Serum ceruloplasmin for Wilson disease Serum alpha-1 antitrypsin RUQ ultrasound Eventual liver biopsy

Treatment of cirrhosis

Type	Recommendations
General	*Slow or reverse progression of liver disease* Abstain from alcohol use Treatment of underlying etiology if known Hepatitis A and B vaccines Yearly influenza vaccination Avoid hepatotoxins (eg, medications, supplements) Adjust medications and dosages for hepatic clearance *Muscle cramps* Replete electrolytes if abnormal Branched chain amino acids 4 g TID Taurine 3 g/day Vitamin E 200 mg TID *Umbilical hernia* Aggressive ascites management Consider surgical repair for refractory patients *Elevated PT/INR or thrombocytopenia* Only treat with plasma for upcoming procedure or active bleeding *Liver transplant* Can be a definitive therapy depending on underlying etiology
Treatment of Ascites	*General* Abstain from alcohol Treat underlying cause of liver disease if known Avoid medications that decrease blood pressure (eg, ARB, ACE) Avoid NSAIDs Avoid beta blockers in refractory ascites Restrict sodium intake to 2 g/day *Diuretics* Spironolactone (Aldactone) 50-100 mg/day Measure urine electrolytes to assess urinary sodium and potassium excretion Add Furosemide (Lasix) 40 mg/day + Spironolactone (Aldactone) 100 mg/day Measure urine electrolytes to assess response and adjust dosage *Refractory ascites* Large volume paracentesis Transjugular intrahepatic portosystemic shunts (TIPS) Liver transplantation
Routine screening/ prevention	*Variceal bleeding* Periodic upper endoscopy for screening for varices Propranolol daily in patients with varices *Hepatocellular carcinoma* RUQ ultrasound every 6 months *SBP* Treat other infections (eg, cellulitis) aggressively to prevent SBP Prophylactic antibiotics during hospitalizations *Hepatic encephalopathy* Avoid triggers Variceal bleeding Infections Sedatives Hypokalemia Hyponatremia Medications Constipation Volume depletion Low protein diet *Hepatorenal syndrome* Avoid nephrotoxic medications
Child-Pugh classification for severity/prognosis	*Ascites* Absent (1 point) Slight (2 points) Moderate (3 points) *Bilirubin* < 2 mg/dL (1 point) 2-3 mg/dL (2 points) > 3 mg/dL (3 points) *Albumin* > 3.5 g/dL (1 point) 2.8-3.5 g/dL (2 points) < 2.8 g/dL (3 points) *INR* < 1.7 (1 point) 1.7-2.3 (2 points) > 2.3 (3 points) *Encephalopathy* None (1 point) Grade 1-2 (2 points) Grade 3-4 (3 points) *Interpretation* Class A (score 5-6) Survival 100% (1 year); 85% (2 year) Class B (score 7-9) Survival 80% (1 year); 60% (2 year) Class C (score 10-15) Survival 45% (1 year); 35% (2 year)

References for cirrhosis

Schuppan D, Afdhal NH. Liver cirrhosis. Lancet. 2008;371(9615):838–851. Link to article
Kockerling D, Nathwani R, Forlano R, Manousou P, Mullish BH, Dhar A. Current and future pharmacological therapies for managing cirrhosis and its complications. World J Gastroenterol2019; 25(8): 888-908. Link to article